User:Renaissancee/AEN

Renaissancee/AEN

Acute esophageal necrosis (AEN), also known as Gurvits Syndrome, Black Esophagus, acute necrotizing esphagitis, and esophageal infarction; is a rare esophageal disorder. AEN defines itself with dark pigmentation of the esophagus, found during an upper gastrointestinal endoscopy. Pigmentation is usually black friable mucosa. The disorder is extremely rare, as only 88 patients over a span of 40 years have received this diagnosis. Specific study of the disorder's mortality rate is mentioned at 31.8%, but new research suggests mortality rates vary from 30-50%. The exact triggering mechanism for this disorder is still unknown, but is likely a multifactorial.

Signs and symptoms

AEN was never recorder as a one symptom disorder, but instead present by multiple symptoms. The symptoms vary from the severity of the disorder. The most classic sign of AEN is the dark pigmentation of esophageal mucosa in an upper endoscopy, usually viewed as an ulcer or as an infectious disease. Necrosis can be found mostly between the three distals of the esophagus, but stops abruptly at the gastroesophageal junction. The basic and most common symptoms reported are coughing and nausea. Left untreated, the coughing, normally phlegm, may become bloody. As more cells become necrotic, the nausea becomes more severe. Severe inflammation may cause breathing difficulties. Upper gastrointestinal bleeding then is reported, and is very commonly represented in elderly patients. The bloody vomiting becomes coffee ground vomiting if left untreated. Epigastrium pain and tenderness, and a mild distended abdomen is common. A cardiovascular event (such as a heart attack) was reported in ten percent of the total known cases.

Risk factors

Modifiable

Having diabetes is currently the most prevalent out of all conditions among patients, with cancer second. High blood pressure is third, due to its weakening of the vessels, can trigger necrosis if severe enough in the esophagus. Chronic lung conditions are another major risk factor. Alcohol abuse weakens the esophagus, to the point where it can become necrotic. Heart disease is also reported to be among one of the top ten preexisting conditions of AEN patients. Malnutrition is also repeatedly reported. Kidney failure either acute or chronic was pronounced.

Nonmodifiable

• Advanced age (average patient aged 57 years old)
• Male sex (4 to 1 male dominance)
• Cirrhosis
• AIDS
• Herpes virus
• Atrial fibrilation

Lesser or unknown of effect

• Aortic dissection
• Anti-cardiolipin antibodies
• CMV infection
• Herpetic infection
• Hyperglycemia
• Hypersensitivity to broad-spectrum antibiotics
• Hypothermia
• Ischemia
• Gastric volvulus
• Posterior mediastinal haematoma
• Septic shock
• Steven Johnson syndrome

Diagnosis and treatment

Diagnosis

Acute esophageal necrosis can only be diagnosed by an upper gastrointestinal endoscopy.

Treatment

Currently, there is no esophageal treatment for AEN. Only treatment is for the underlying main diseases or conditions. Appropriate hydration is set. Antacids are also added for further recovery support. Common support drugs of antacids are either H₂ receptor antagonists, and/or a proton pump inhibitor. Sucralfate was used as an option or "last resort" drug, and used along with parenteral nutrition greatly increased chance of recovery. An esophagectomy can be issued if the disorder is severe enough.

In popular culture

Acute esophageal necrosis made an appearance on an American medical drama show, Dr. G: Medical Examiner. Jan Garavaglia, the show's host, receives a female body, that at time of the autopsy had a severe case of acute esophageal necrosis due to chronic alcoholism.

History

Acute esophageal necrosis was first described by Goldenberg et al in 1990. Cases have emerged since 1960, but have never been described as the common names for AEN. Due to it's rarity, only 88 cases have been pronounced AEN, but most likely there is much more. Gurvits et al describes AEN as "poorly described in medical literature".